Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many people living with cystic fibrosis and their families face complicated issues related to getting the care they need. Cystic Fibrosis Foundation Compass makes sure that no one has to do it alone.
CF Foundation Compass is a service that helps people with CF and their families with navigating insurance options, connecting to legal information and experts, finding available financial resources, and tackling other life issues.
CF care team members are paramount in providing highly specialized care to people living with CF. CF Foundation Compass can help by serving as a strategic ally for care teams, so team members can focus on their patients’ care.
CF Foundation Compass can help you navigate insurance, financial, legal, and other issues you are facing. Use this online form to start your conversation with a Compass case manager today.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
Substance misuse is the use of alcohol, illegal drugs, or over-the-counter or prescription medications in a way that they are not meant to be used. Over time, substance misuse can become an unhealthy way to respond to stress and anxiety, particularly when managing a chronic illness like cystic fibrosis.
Substance misuse is the use of alcohol, illegal drugs, or over-the-counter or prescription medications in a way that they are not meant to be used and could be harmful to you or others around you. People can misuse substances one time, occasionally, or regularly, and they can go on to develop substance use disorder.
Substance use disorder occurs when the use of alcohol or drugs impairs your health or how you function in your daily life. Examples of this kind of impairment include problems with physical or emotional health, disability, and failure to meet major responsibilities. Substance use disorders can be mild, moderate, or severe.
The terms “substance misuse” and “substance use disorder” reflect changing attitudes about substance use. People may be more familiar with the terms “substance abuse,” “dependence,” or “addiction.” Many people associate these terms with the view that using substances is a moral failing. However, this view is beginning to change. We are learning that misusing substances regularly can change the way the brain functions. As a result, a substance use disorder is now increasingly being understood as a long-term brain disease, and as a medical issue instead of a moral one.
Substances can be anything you put into your body that can change how your brain functions. These changes can affect your:
Commonly misused substances include:
Sydney Sabol, an adult with CF, discusses how substance misuse is more common than people think and why it should be treated like any other illness.
Misuse isn't necessarily limited to one substance. Polysubstance use refers to taking more than one substance at the same time. This can be intentional, such as mixing a drug with alcohol to enhance the effect. It can also be unintentional, such as drinking alcohol without realizing that it will interfere with a prescription medication or taking two prescription medications without knowing how they interact.
Substance misuse can lead to problems with your physical health, your emotional health, and your social and financial well-being. Even casual substance misuse can have serious consequences, such as motor vehicle accidents, violence toward others, alcohol poisoning, and overdose. Research suggests that substance misuse is at least as common among people with cystic fibrosis as it is in the general population and it can negatively affect the disease.
Daily CF treatments are an important part of slowing disease progression, and misusing substances can make it harder to sustain daily care. One study that looked at alcohol and opiate misuse among adults with CF found that those who misused alcohol or opioids were more likely to skip medical appointments and were more frequently hospitalized than people with CF who did not misuse alcohol or opiates. And although they did not experience depression or anxiety at a higher rate, their depression and anxiety was twice as severe compared to those who did not misuse these substances.1 Another study of high school students with chronic illnesses found that those who used alcohol were more likely to forget to take, or intentionally skip taking, their medications than those who did not drink.2
Substance misuse can also worsen existing conditions associated with CF. For example, misuse of drugs like cocaine, opiates, and methamphetamines can cause weight loss, while smoking tobacco and inhaled marijuana causes lung damage. In addition, smoking marijuana can introduce germs, such as bacteria and fungi, into the airways. People who misuse alcohol can damage their livers and pancreases, develop high blood pressure or cancer, and increase their risk of a stroke.
Moreover, drug-to-drug interactions that result from inappropriate use of substances can be dangerous or reduce the effectiveness of CF medications. For example, people who have received a lung transplant take medications to suppress their immune systems to prevent organ rejection. Some of the active ingredients in marijuana can interfere with how these medications work. The active ingredient in marijuana, as well as other medications, can also affect how cystic fibrosis transmembrane conductance regulator (CFTR) modulators work.
Medical marijuana has become more commonly prescribed, but it has not been tested in the same way as other prescription medications approved by the U.S. Food and Drug Administration (FDA). It is important to remember to tell your CF care team that you are taking it. A member of your care team or your pharmacist can tell you if any substances you take might affect your CF medications.
It also is important that your CF care team knows all the prescription or non-prescription substances you are taking because some substance misuse and withdrawal symptoms, such as fatigue, low appetite, weight loss, vomiting, and diarrhea, can overlap with CF symptoms. This can make it difficult for your CF care team to provide the most appropriate care.
Substance use disorders can impact close friends and family, who may object to any amount of substance misuse. In the case of substance use disorders, it can have a particularly large emotional and financial impact on them. Those close to a person who misuses substances can have feelings that include anger, helplessness, depression, and anxiety. It is important that close friends and family seek help coping with their loved one's substance misuse if they need to.
Caring for or about somebody with CF is stressful, and it can be more stressful if you have a loved one who misuses substances. CF and Mental Health: Building Resilience can help you deal with this stress before it becomes overwhelming.
Relationships and your ability to study, work, or manage finances can also suffer because substance misuse can affect your mood, thinking, and behavior. More severe forms of substance misuse can affect relationships in several ways and lead to isolation.
Although it varies from center to center, substance misuse may affect eligibility for a transplant. Many transplant centers consider current substance misuse, including tobacco use, as a reason not to list a person for transplant. Others will accept people who have stopped using substances for a while. Each case is considered individually.
If you're concerned that you may not qualify for a transplant because of substance misuse, talk to a member of your care or transplant team. They can help you address it. For example, some centers will agree to list a person after the person has proven he or she has avoided substance misuse for a period of time. It is important to be honest with your care team so they can give you as much support as possible.
People with cystic fibrosis can begin misusing substances for many of the same reasons people without CF do. This includes experimentation or wanting to fit in with peers, especially during times of transition, like starting college. But, CF can also be at the root of a person's substance misuse. Some people with CF misuse substances as a response to:
Sydney Sabol, an adult with CF, discusses how isolation during her teen years led her to substance misuse.
People with CF who also have other conditions, such as attention deficit hyperactivity disorder (ADHD), bipolar disorder, or obsessive-compulsive disorder (OCD), may use substances to “self-medicate,” especially if they have not been diagnosed and properly treated for these conditions. For example, people may misuse substances, such as alcohol, marijuana, or opiates, to try to get to sleep or to calm racing thoughts and restlessness. These attempts at self-medication could interfere with CF medications or the effectiveness of other treatments, such as medications used to treat conditions like depression or anxiety.
"And of course, high-dose medications, such as opiates, need to be used judiciously where there is chronic pain … As patients build a tolerance to pain medications and require higher and higher doses, their pain eventually will be difficult or impossible to adequately and safely manage … there is a very real risk of addiction and drug misuse." -- Joey Disser, adult with CF, from the CF Community Blog.
For people with cystic fibrosis, even casual misuse of substances can have serious health consequences, especially if it interferes with daily life.
Many people use substances socially and do not develop a problem with substances. Others find that they need more of the substance to experience the same effect. Some people may start misusing substances socially but find that, over time, they begin to use substances to cope with stress, depression, or anxiety. Gradually, they may develop a substance use disorder instead of practicing effective self-care. Learn more about how you can help yourself cope with depression and anxiety.
Signs of a substance use disorder include:
The National Institute on Drug Abuse has several questionnaires that you can take if you think you or a friend or loved one may have a substance misuse problem. Your care team can also help you find resources that screen for substance misuse issues.
Preventing substance misuse before it becomes a problem can start with letting members of your care team know if you're finding it harder to manage daily life, including doing your treatments, or if you're experiencing feelings of depression or anxiety.
Prevention is especially important in the case of children and adolescents. Children who try alcohol before age 14 are more likely to develop substance misuse issues at some point in their lives compared to those who do not drink alcohol until age 21. Knowing what factors increase or decrease a person's risk for developing a substance misuse issue is an important part of prevention. A member of your or your child's care team can help you find substance misuse prevention resources.
People are at higher risk for substance misuse during transitions, including:
Certain factors increase a child or adolescent's risk for substance misuse, including:
Factors that decrease a child or adolescent's risk for substance misuse include:
Although peers, schools, and belonging to a community all play a role in preventing substance misuse, parents may play the most important role by setting boundaries, starting with clearly stated rules, the consequences for breaking those rules, and communicating the risks associated with substance misuse.
Parents can learn more about talking to their teens about substance misuse at childmind.org. The Substance Abuse and Mental Health Services Administration also has substance misuse prevention resources, as does the American Academy of Child and Adolescent Psychiatry.
“As parents, medical professionals, and other caring adults who are involved with children and teens with chronic conditions, we can make a life-transforming difference by focusing as much on their mental health as we do on their physical health.” -- Lisa C. Greene, mother of children with CF
Read "5 Ways Parents Can Foster Hope in CF Teens."
As role models for their children, parents can practice self-care and resilience-building to address their own stress, particularly as caregivers to children with CF. A large study showed that depression and anxiety are greater among people who care for children with CF than the general population.3
Recognizing you have a problem is the first step. If you think you may be misusing a substance, let a member of your cystic fibrosis care team know. They can help you identify the underlying causes of your misuse, as well as triggers for substance misuse behaviors.
By starting the conversation, your care team can help you figure out what may be contributing to your stress. They can partner with you to find possible solutions, including ways to practice self-care and build resilience to cope with setbacks or changes in your life.
Admitting to someone that you may have a substance misuse problem takes courage. To help with the conversation, you might tell a member of your care team with whom you have a good relationship. It might also help to bring a person you trust for support.
If you don't want to talk to your CF care team, you can speak with your primary care physician. You can also try the Substance Abuse and Mental Health Services Administration (SAMHSA) national helpline at 800-662-HELP (800-662-4357). If you feel you need help with substance misuse, the important thing is to be honest and to keep asking until you get the help you need.
Here are some ways to start the conversation with a member of your care team:
Not everybody feels ready or able to stop misusing substances entirely. You can ask your care team about ways you can lower the risks to your health, including:
Many people hesitate to talk to their care teams because they are afraid of being judged or denied care. They may not even realize the effects of their substance misuse on their health due to things like drug-to-drug interactions. It is important to remember that you're more likely to get the right care if you start the conversation.
“When my nurse coordinator mentioned therapy, I was hesitant. I told myself, and her, that I wasn't crazy (because only crazy people go to therapy?) and that I could get through this, just like I had gotten through everything else. But the truth was I couldn't get through it alone, and once I realized that, it was like a huge weight had been lifted off my shoulders.” Jessika Allison, adult with CF
Read more about Jessika's experience.
There are many options for substance misuse treatment, and whatever you decide should be tailored for your unique needs, such as how severe your substance misuse is. Treatment for substance misuse includes:
Your care team, or the substance misuse expert your care team refers you to, should help develop a recovery plan that works for you.
Anne Clark, whose daughter Kate had CF and substance misuse issues, talks about the importance of care teams treating mental health issues as well as CF.
Behavioral treatment (talk therapy) has been shown to help people with substance misuse issues. Many people find working with a health professional to identify and change the behaviors that lead to substance misuse can help. This includes:
Behavioral treatment can also help address other emotional issues, such as anxiety or depression.
Your doctor can also prescribe medications to help you stop misusing substances, including:
Addiction treatment also includes long-term follow-up to prevent relapse.
In addition to medical and behavioral treatments, many people have found success recovering from addiction by joining a peer support program, such as a 12-step recovery program. Almost all 12-step programs are modeled on Alcoholics Anonymous. Twelve-step recovery programs include:
For people who feel the structure and methods of 12-step programs are not a good fit for them, there are also other effective recovery programs. Several mobile applications are available that can help in substance use disorder recovery.
Addiction can be a long-term condition that may include slips and relapses. A slip is a brief return to misusing substances. A person might drink or use drugs once but stop soon after. A relapse is a return to misusing substances after a period of recovery.
Many people experience a sense of failure after a slip or relapse, but these setbacks do not need to end a person's intention to stay sober. In fact, it could motivate a person even more to sustain their recovery.
Many experts are starting to see substance use disorders as a chronic, long-term condition much like high blood pressure or diabetes, which are diseases that flare up and need treatment. If you do experience a slip or relapse, the important thing is to be honest with yourself and your support network, which can be your family, your 12-step sponsor, your counselor, members of your care team, or an online support group.
Practicing healthier coping skills so that they are habit-forming can reduce the probability of slips and relapses. Studies of tobacco smokers have shown that the more a person tries or practices the act of not smoking by replacing the urge with a less harmful activity, the more likely they are to ultimately quit smoking in the long term.
No matter how effective substance misuse treatment is, there will still be aspects of life that people who don't have CF won't understand. CF Peer Connect is a peer mentoring program for people with CF and their family members age 16 and older. Talk with and learn from someone who is also affected by CF and has gone through similar experiences.
One of the best ways to prevent relapse is to know the signs. Typically, that means understanding what triggers you to misuse substances. Triggers are things that cause cravings for the substance in your brain. They can include:
Often, simple acts that divert you from thinking about the trigger can help the craving pass. For example, after identifying triggers, you can react to them in a healthy way, such as:
Most health insurance policies cover treatment related to substance misuse. Specific coverage depends on if your insurance is private, public, or if you have no insurance. CF Foundation Compass case managers can assist you with identifying local resources based on the substance being used and the community programs available.
If you need help with substance misuse treatment, you can contact Compass, a free and personalized service that can help you with insurance, financial, legal, and other issues. Case managers can assist with identifying local resources, as well as help you identify your benefits and put you in touch with resources.
Contact CF Foundation Compass at:
Monday through Friday, 9 a.m. until 7 p.m. ET
Reference to any specific product, process, or service does not necessarily constitute or imply its endorsement, recommendation, or favoring by the Cystic Fibrosis Foundation. The appearance of external hyperlinks does not constitute endorsement by the Cystic Fibrosis Foundation of the linked websites, or information, products, or services contained therein.
Information contained on this site does not cover all possible uses, actions, precautions, side effects, or interactions. This site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.
FDA-approved drug information is available at dailymed.nlm.nih.gov/dailymed.
1. Richards CJ, Pinsky H, Yonker LM, Friedman D, Georgiopoulos AM. Alcohol and Opiate Misuse in Adults with Cystic Fibrosis [NACFC 2018 Accepted Abstract]. (Back to text)
2. Weitzman ER, Ziemnik RE, Haung Q, Levy S. Alcohol and Marijuana Use and Treatment Nonadherence Among Medically Vulnerable Youth. Pediatrics. 2015 Sep;136(3);450-7. (Back to text)
3. Quittner AL, Goldbeck L, Abbott J, et al. Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: results of The International Depression Epidemiological Study across nine countries. Thorax. 2014 Dec;69(12):1090-7. (Back to text)
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